We report a case of a 7-year-old child who required emergency surgery for acute abdomen and suspected acute appendicitis. During surgery a tumor located in the small bowel that caused intestinal occlusion was found. Histopathologic analysis showed a solitary gastrointestinal myofibroma. This is a very rare tumor, especially as a single lesion, because in world literature, there are less than 10 cases reported.
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http://dx.doi.org/10.1016/j.jpedsurg.2005.05.031 | DOI Listing |
Diagnostics (Basel)
December 2021
"Sfânta Maria" Emergency Children Hospital Iași, 700309 Iași, Romania.
Background: Infantile myofibromatosis (IM) is a soft tissue disease with solitary or multiple benign tumors, and an etiology still unknown. IM is a mesenchymal disorder of early infancy and is more frequent in males. IM may present as a solitary lesion of the skin, bone, muscle, subcutaneous tissue, located at the head, neck, and trunk, with good prognosis; or, as a multicentric form, with or without visceral involvement (heart, lung, gastrointestinal tract, kidney), with a poor prognosis.
View Article and Find Full Text PDFUrology
August 2021
Section of Pediatric Urology, Children's Healthcare of Atlanta and Emory University School of Medicine, Atlanta, GA.
Inflammatory myofibroblastic tumors (IMT) are rare and poorly understood inflammatory neoplasms. Most commonly occurring in the liver and gastrointestinal tract, cases of bladder involvement have been rarely reported. Bladder IMT generally presents with gross hematuria and can be differentiated from other bladder tumors by expression of anaplastic lymphoma kinase.
View Article and Find Full Text PDFSurg Case Rep
January 2021
Division of Pediatric Surgery, Niigata University Graduate School of Medical and Dental Sciences, 1-757 Asahimachi-dori, Chuo-ku, Niigata City, Niigata, 951-8510, Japan.
Background: Intestinal obstruction caused by a tumor is very rare in newborns, and the preoperative diagnosis is difficult. We herein report a rare case of neonatal colonic obstruction due to solitary intestinal myofibroma with characteristic findings on gastrografin enema and the surgical strategy.
Case Presentation: A 4-day-old female infant presented to our neonatal intensive-care unit with abdominal distention and bilious vomiting after feeding.
Cell Mol Life Sci
April 2021
De Duve Institute, Université Catholique de Louvain, Avenue Hippocrate 75, Box B1.74.05, 1200, Brussels, Belgium.
PDGFRA and PDGFRB are classical proto-oncogenes that encode receptor tyrosine kinases responding to platelet-derived growth factor (PDGF). PDGFRA mutations are found in gastrointestinal stromal tumors (GISTs), inflammatory fibroid polyps and gliomas, and PDGFRB mutations drive myofibroma development. In addition, chromosomal rearrangement of either gene causes myeloid neoplasms associated with hypereosinophilia.
View Article and Find Full Text PDFRadiol Case Rep
November 2020
Department of Pediatrics, University of Maryland School of Medicine, 110 S. Paca St., 8th Floor Neonatology, Baltimore, MD 21201.
Infantile myofibromatosis, the most common fibrous tumor of infancy, is classified in 2 forms; as a solitary nodule or as numerous, widely-distributed multicentric lesions with or without visceral involvement. Although benign, multicentric myofibromas are still associated with a high incidence of morbidity and mortality due to the infiltration of critical structures. Herein, we present a case of an infant with aggressive and mutation-negative myofibromas distributed throughout the vascular, respiratory, and gastrointestinal systems.
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